Multiple Metastatic Leiomyosarcoma of the Pancreas: A First Case Report and Review of the Literature

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1 CASE REPORT Multiple Metstti Leiomyosrom of the Pnres: A First Cse Report nd Review of the Literture Tkeshi Ogur 1, Disuke Msud 1, Yoshitk Kurisu 2, Yoshihru Miymoto 3, Mihihiro Hyshi 3, Akir Imoto 1, Mihiki Tkii 1, Toshihis Tkeuhi 1, Tkuy Inoue 1, Stoshi Tokiok 1, Kzuhis Uhiym 3, Eiji Umegki 1 nd Kzuhide Higuhi 1 Astrt A 60-yer-old womn ws referred to our hospitl with swelling of the right leg. After surgery, leiomyosrom of the right leg ws dignosed. Computed tomogrphy showed two hypovsulr msses in the pnreti ody nd til tht were heterogeneously enhned ompred with the pnreti prenhym. On endosopi ultrsonogrphy, the tumors in the pnreti ody nd til oth exhiited regulr mrgins nd were visulized s well-irumsried msses with uneven interiors. Distl pnretetomy ws performed under presumptive dignosis of metstti pnreti leiomyosrom dignosed sed on the findings of EUS- FNA. On lprotomy, peritonel wshing ytology yielded negtive results, nd no dissemintion ws oserved. Ultimtely, metstti pnreti leiomyosrom ws histologilly dignosed. Key words: pnres, EUS-FNA, metstti tumor, multiple () () Introdution The inidene of metstti pnreti tumors hs een reported to e only 1.6-3% (1). Lung, renl nd gstri ners hve een reported to e the most ommon primry tumors ssoited with pnreti metstsis (2-4). Leiomyosrom is rre soure of pnreti metstsis ompred to these tumors. Pnreti metstti tumors re usully seen in ptients with disseminted disese nd metstses in other orgns nd re primrily deteted on utopsy. Solitry metstsis to the pnres is thus extremely rre. To the est of our knowledge, only 25 ses hve een reported to dte (1, 2, 5-25). In ddition, no ses of multiple metstti leiomyosrom in the pnres without dissemintion or other metstti orgns hve een previously reported. We herein report the first se of multiple metstti leiomyosrom in the pnres without dissemintion or other metstti orgns nd review previously pulished reports. Cse Report A 60-yer-old womn ws referred to our hospitl with swelling of the right leg. The mss in the right leg mesured 50 mm in mximum dimeter nd ws heterogeneously enhned on omputed tomogrphy (CT) (Fig. 1). Leiomyosrom of the right leg ws dignosed following exmintions performed in the deprtment of orthopedi surgery. In ddition, preopertive 18 F-fluorodeoxygluose (FDG)-positron emission tomogrphy (PET) showed strong umultion in the pnreti ody nd til (Figs.1-d). Surgery of the right leg reveled the presene of leiomyosrom. Three weeks fter surgery, the ptient ws referred to our deprtment of internl mediine for further exmintions of the pnreti msses. All lortory vlues were norml, inluding the serum levels of rohydrte ntigen 19-9 nd rinoemryoni ntigen, with the exeption of slight elevtions in the levels of mylse (153 ng/dl), elstse-1 (726 ng/dl) nd C-retive protein (0.55 mg/dl). CT showed two The 2nd Deprtment of Internl Mediine, Osk Medil College, Jpn, The 2nd Deprtment of Pthology, Osk Medil College, Jpn nd Deprtment of Gstroenterogil Surgery, Osk Medil College, Jpn Reeived for pulition July 13, 2012; Aepted for pulition Novemer 28, 2012 Correspondene to Dr. Tkeshi Ogur, ogurtkeshi0411@yhoo.o.jp 561

2 d Figure 1. CT showed heterogeneously enhned mss in the right leg (), nd FDG-PET showed strong umultion (). In ddition, the pnreti ody () nd til (d) exhiited strong umultion. Figure 2. CT showed hypovsulr msses in pnreti ody nd til tht were heterogeneously enhned ompred with the pnreti prenhym (). The min pnreti dut ws stenosed, mesuring 10 mm, in the pnreti ody, nd dilttion mesuring 5 mm in mximl dimeter ws seen in the til (). hypovsulr msses mesuring 30 mm nd 70 mm in mximum dimeter in the pnreti ody nd til tht were heterogeneously enhned ompred with the pnreti prenhym. In ddition, neroti nd ysti hnges were seen in oth msses (Fig. 2). Mgneti resonne imging (MRI) showed hyperintensity of the pnreti msses on T2- weighted imging nd deresed diffusion on diffusionweighted imging. The min pnreti dut ws stenosed, 562

3 Figure 3. The tumors in the pnreti ody () nd til () oth exhiited regulr mrgins nd were visulized s well-irumsried msses with uneven interiors. On ontrst-enhned EUS (), mrked enhnement ws oserved from the erly stge of enhnement, nd n nehoi region suggesting the presene of nerosis ws seen in the interior. EUS-FNA ws performed using 22G needle (rrow). mesuring 10 mm, in the pnreti ody, nd dilttion mesuring 5 mm in mximl dimeter ws seen in the til on mgneti resonne holngiopnretogrphy (MRCP) (Fig. 2). On endosopi ultrsonogrphy (EUS), the tumors in the pnreti ody nd til oth exhiited regulr mrgins nd were visulized s well-irumsried msses with uneven interiors. The tumors were not ontinuous with eh other. On ontrst-enhned EUS using Sonzoid (Diihi- Snkyo, Tokyo, Jpn), mrked enhnement ws oserved from the erly stge of enhnement, nd n nehoi region suggesting the presene of nerosis ws seen in the interior. Smples otined from the pnreti ody nd til msses using EUS-guided fine needle spirtion (FNA) reveled histopthologil prolifertion of typil spindleshped ells. Immunohistohemil stining demonstrted tht these ells were positive for α-sma nd vimentin nd negtive for CD34 nd desmin (Figs. 3, 4). These findings were extremely similr to the histologil findings of leiomyosrom in the leg. Distl pnretetomy ws performed under presumptive dignosis of metstti pnreti leiomyosrom. On lprotomy, peritonel wshing ytology yielded negtive results, nd no dissemintion ws oserved. In the reseted smples, elsti hrd tumors with smooth surfe were identified in the pnreti ody s well s the pnreti til. The setions lso exhiited solid, gryish-white tumors with psules. The interior ontined vity suggestive of nerosis (Fig. 5). Histopthologilly, hemtoxylin nd eosin (HE) stining showed fsiulr prolifertion of typil spindle-shped ells with prtil invsion into the pnreti dut. Immunostining reveled the tumors to e CD34-negtive, desmin-negtive, α-smapositive nd vimentin-positive. As these findings were very similr to those of the leiomyosrom in the thigh, metstti pnreti leiomyosrom ws dignosed (Fig. 6). No signs of reurrene hve een oserved s of one yer postopertively. Disussion Metstti pnreti tumors re rre. In series of 4,995 dult utopsies, Adsy et l. reported only 81 ses (1.6%) of metstti pnreti tumors (1). Most of these ses involved disseminted rinom nd other metstti orgns; therefore, isolted pnreti metstses re even more rre. Rolnd et l. (26) reported tht only 27 of 1,357 ptients (2%) with solitry pnreti msses hd metstti pnreti tumors, only four of whih were reseted. In ddition, Nkmur et l. (2) reviewed the utopsy reords nd pthologil fetures of 103 ses of metstti pnreti tumors nd found tht gstri ner ws the most ommon primry 563

4 Figure 4. Smples otined from the pnreti ody nd til msses using EUS-FNA reveled histopthologil prolifertion of typil spindle-shped ells ( 100, ). Immunohistohemil stining demonstrted tht these ells were positive for α-sma ( 100, ) nd vimentin ( 100, ) nd negtive for CD34 nd desmin. Figure 5. Elsti hrd tumors with smooth surfe were identified in the pnreti ody s well s the pnreti til. The interior ontined vity suggestive of nerosis. tumor site (20%), followed y the lungs (18%) nd extrhepti ile dut (13%), with leiomyosrom representing rre primry tumor (<2%). To the est of our knowledge, metstti pnreti leiomyosrom hs een reported previously in only 25 ses (1, 2, 5-25). Moreover, isolted metstti pnreti leiomyosrom without dissemintion or metstses in other orgns is extremely rre. Tle shows review of the 25 previously reported ses of metstti pnreti leiomyosrom. Among these ses, the men ptient ge t the time of dignosis ws 51.7 yers (rnge: yers) nd the rtio of men to women ws 6:14. The uterus ws the most ommon primry tumor site (n=7), followed y the legs (n=5), retroperitoneum (n=3) nd gstrointestinl trt (n=3). The pnreti hed ws the most frequent site of metstsis, nd lmost ll lesions represented solitry metstti pnreti leiomyosrom. On the other hnd, few ses involved multiple metstses to the pnres. However, these ses lso involved other metstti orgns or dissemintion. In the present se, despite the presene of multiple metstti pnreti lesions, no other metstti orgns or dissemintion were identified. This my represent the first report of suh se. When metstti tumors of the pnres re solitry, mking preopertive differentil dignosis etween primry pnreti tumor nd metstti tumor remins hllenging. The symptoms of pnreti metstti tumors ommonly inlude ostrutive jundie, dominl pin nd ody weight loss, while new-onset dietes is oserved in ses of primry pnreti tumors (19, 27). However, pnreti metstti tumors re frequently symptomti nd deteted only inidentlly during follow-up. On the other hnd, imging tehniques suh s CT nd MRI my support the linil suspiion of metstti tumors to the pnres, prtiulrly when multiple lesions re deteted in the pnres (28). Z grggen et l. (27) reported the ses of 10 ptients with isolted metstses in the pnres, mong whom the tumors were initilly misdignosed s pnreti ner in seven of the ses. Detiled nmnesis, linil spets, FNA, onogene nlyses suh s K-rs muttion nlyses (29) nd imging findings re thus ll importnt for the urte dignosis nd tretment of pnreti metstsis (30). In generl, the time etween dignosis of primry ner nd detetion of pnreti metstsis vries widely, rnging from 0.5 to 2 yers in ptients with lung ner, 7.5 yers in ptients with rest ner nd 10 yers in ptients with renl ell rinom (31). Likewise, in reported ses of leiomyosrom, the intervl to metstsis or reurrene vries widely from pproximtely one to 10 yers. As suh, ses of slow-growing pnreti metstses hve een reported; however, the detils of these metstses hve not een eluidted. Regrding the prognosis of metstti pnreti tumors, Rddy et l. (32) reported 5-yer survivl rte of 36-88% in ptients undergoing resetion, representing more fvorle rte thn tht of ptients with pnreti dutl denorinom, with prognosis tht improves with inresing time to reurrene. A similr pttern hs een oserved for leiomyosrom, with fvorle prognosis eing reported 564

5 Figure 6. Histopthologilly, Hemtoxylin nd Eosin stining showed fsiulr prolifertion of typil spindle-shped ells ( 200, ). Immunostining reveled the tumors to e CD34-negtive, desmin-negtive, α-sma-positive ( 200, ) nd vimentin-positive. These findings were very similr to those of the leiomyosrom in the thigh ( 200, ). Tle. Reports of Metstti Leiomyosrom of the Pnres Author Yer Age / Sex Symptom Primry Other metstti orgns Lotion Numer of msses Tretment Outome Werneke K / M Asymptomti Spermti Lung, one, skin Multiple Multiple n.d n.d ord Werneke K / F Asymptomti olon liver Pt 1 n.d n.d Murnk T 1986 n.d n.d n.d n.d n.d n.d n.d n.d Akt D / F n.d n.d Thyroid, one, Ph, P 2 n.d n.d slivry glnd Le Borgne J / F n.d vein n.d n.d n.d PD 40mont h Ded Nsu M / F n.d ovry Liver, lung, n.d n.d n.d Autopsy stomh, musle Nkmur E 2001 n.d n.d Retroperitone n.d n.d n.d n.d Autopsy l tissue Nkmur E 2001 n.d n.d Soft tissue n.d n.d n.d n.d Autopsy Ishigure K / M n.d Suutneous tissue Sperti C / M Gstrointestinl leeding n.d Ph 1 PD 36mont duodenum olon Ph 1 PPPD 42mont h Ded Adsy VN 2004 n.d n.d Retroperitone n.d n.d n.d n.d n.d l tissue Iwmoto I / F Asymptomti Uterine Lung P 1 DP 8month Alive Nkjim Y / M Asymptomti Left lower leg Lung, gstri Ph 1 None 7month Ded Cripp S / M Jundie Jejunum Liver Ph 1 PPPD 56mont Floni M / F Asymptomti Uterine Liver Ph 1 PPPD Alive Koh YS / F Asymptomti Right thigh None Ph 1 PD Alive Burke JP / M Mlise, et Anterior right None Ph 1 PD n.d thigh Skgis L / F Asymptomti Uterine n.d n.d n.d Prtil n.d pnretetomy Clemente G / F Jundie Uterine None Ph 1 PD n.d Alonso Gome J / F Asymptomti Uterine Gllldder Ph 1 Segmentl pnretetomy Hernndez S / F Asymptomti Uterine Liver, lung, Ph 1 PD Alive dissemintion, et Ll H / F Asymptomti Retroperitone Liver, lung, Ph 1 None n.d l tissue dissemintion, et Ko YH 2011 n.d n.d Uterine Lung Ph 1 PD n.d You DD / F Asymptomti Lower extremity 18mont None Ph 1 PPPD 21mont Roert PE / F Asymptomti Left thigh Ri Pt 1 DP 10mont n.d: not disussed, Ph: pnreti hed, P: pnreti ody, Pt: pnreti til, PD: Pnretoduodenetomy, PPPD: Pyloru-preserving pnretoduodenetomy 565

6 for ptients undergoing resetion. In onlusion, we herein report the first se of multiple metstti leiomyosrom to the pnres without dissemintion or other metstti orgns. In ses of solitry metstsis with no other distnt metstses or dissemintion, tive resetion is onsidered importnt for hieving fvorle prognosis in the sene of other effetive ntiner gents, even in ses of multiple tumors, suh s the present se. The uthors stte tht they hve no Conflit of Interest (COI). Referenes 1. Adsy NV, Ande A, Bsturk O, Kilin N, Nssr H, Cheng JD. Seondry tumors of the pnres: n nlysis of surgil nd utopsy dtse nd review of the literture. Virhows Arh 444: , Nkmur E, Shimizu M, Itoh T, Mne T. Seondry tumors of the pnres: liniopthologil study of 103 utopsy ses of Jpnese ptients. Pthol Int 51: , Hiotis SP, Klimstr DS, Conlon KC, Brennn MF. Results fter pnreti resetion for metstti lesions. Ann Surg Onol 9: , Rolnd CF, vn Heerden JA. Nonpnreti primry tumors with metstsis to the pnres. Surg Gyneol Ostet 168: , Roert PE, Orry D, Mor C, et l. Resetle pnreti metstsis of left thighone leiomyosrom: se report nd literture review. J Gstrointest Cner 43: 40-43, You DD, Choi DW, Choi SH, et l. Surgil resetion of metstsis to the pnres. J Koren Surg So 80: , Ko YH, Sd U, Tn AE, Mgsomol BM, Pdhy AK. Fluorine- 18-fluorodeoxygluose PET/CT for the evlution of suspeted reurrent uterine leiomyosroms. At Rdiol 52: , Ll H, Neyz Z, Kpoor VK, Pottkkt B, Gupt P. Lol reurrene nd multi-orgn metstsis of primry retroperitonel leiomyosrom in unusul lotions fter surgil resetion. J Rdiol Cse Rep 5: 1-8, Hernández S, Mrtín-Fernández J, Ls I, Busteros I, Grí- Moreno F. Pnretioduodenetomy for metstsis of uterine leiomyosrom to the pnres. Clin Trnsl Onol 12: , Alonso Gómez J, Arjon Sánhez A, Mrtinez Ceilli D, et l. Uterine leiomyosrom metstsis to the pnres: report of se nd review of the literture. J Gstrointest Cner 43: , Clemente G, Giordno M, De Rose AM, Nuzzo G. Imge of the month. Metstsis from leiomyosrom in the hed of the pnres. Arh Surg 145: , Skgis L, Iouli E, Politi E. Isolted pnreti metstsis of uterine leiomyosrom dignosed y fine needle spirtion iopsy. At Cytol 54: , Burke JP, Mguire D, Dillon J, Morirty M, O Toole GC. Whipple s proedure for n oligometstsis to the pnres from leiomyosrom of the thigh. Ir J Med Si 181: , Koh YS, Chul J, Cho CK, Kim HJ. Pnreti metstsis of leiomyosrom in the right thigh: se report. World J Gstroenterol 13: , Floni M, Cripp S, Srgenti M, Cpelli P, Pederzli P. Pnreti metstsis from leiomyosrom of the rod ligment of the uterus. Lnet Onol 7: 94-95, Cripp S, Angelini C, Mussi C, et l. Surgil tretment of metstti tumors to the pnres: single enter experiene nd review of the literture. World J Surg 30: , Nkjim Y, Kkizki S, Knd D, et l. Pnreti nd gstri metstses of leiomyosrom rising in the left leg. Int J Clin Onol 10: , Iwmoto I, Fujino T, Higshi Y, et l. Metstsis of uterine leiomyosrom to the pnres. J Oset Gyneol Res 31: , Sperti C, Psquli C, Liessi G, Pinirdi L, Deet G, Pedrzzoli S. Pnreti resetion for metstti tumors to the pnres. J Surg Onol 83: , Ishigure K, Kneko T, Tked S, Inoue S, Kwse Y, Nko A. Pnreti metstsis from leiomyosrom in the k. Heptogstroenterology 50: , Nsu M, Inoue J, Mtsui M, Minour S, Mtsur O. Ovrin leiomyosrom: n utopsy se report. Pthol Int 50: , Le Borgne J, Prtensky C, Glemin P, Dups B, de Kerviller B. Pnretioduodenetomy for metstti mpullry nd pnreti tumors. Heptogstroenterology 47: , Akt D, Arlsmk A, Ozmen MN, Akhn O, Altund K, Güllü I. US nd CT findings of multientri leiomyosromtosis. Eur Rdiol 9: , Murnk T, Teshim K, Hond H, Nnjo T, Hnd K, Oshiumi Y. Computed tomogrphy nd histologi pperne of pnreti metstsis from distnt soures. At Rdiol 30: , Werneke K, Peters PE, Glnski M. Pnreti metstses: US evlution. Rdiology 160: , Rolnd CF, vn Heerden JA. Nonpnreti primry tumors with metstsis to the pnres. Surg Gyneol Ostet 168: , Z grggen K, Fernández-del Cstillo C, Rttner DW, Sigl H, Wrshw AL. Metstses to the pnres nd their surgil extirption. Arh Surg 133: , Boudghène FP, Deslndes PM, LeBlnhe AF, Bigot JM. US nd CT imging fetures of intrpnreti metstses. J Comput Assist Tomogr 18: , Ogur T, Ymo K, Swki A, et l. Clinil impt of K-rs muttion nlysis in EUS-guided FNA speimens from pnreti msses. Gstrointest Endos 75: , Hiotis SP, Klimstr DS, Conlon KC, Brennn MF. Results fter pnreti resetion for metstti lesions. Ann Surg Onol 9: , Minni F, Csdei R, Perenze B, et l. Pnreti metstses: oservtions of three ses nd review of the literture. Pnretology 4: , Rddy S, Wolfgng CL. The role of surgery in the mngement of isolted metstses to the pnres. Lnet Onol 10: , The Jpnese Soiety of Internl Mediine 566